title: A rare occurrence of pulmonary alveolar proteinosis after lung transplantation.
abstract: We present a case of pulmonary alveolar proteinosis (PAP) initially diagnosed 28 months after left single-lung transplantation for idiopathic pulmonary fibrosis. The diagnosis was based upon the presence of periodic acid-Schiff (PAS)-positive and surfactant immunostain-positive acellular lipoproteinaceous material within alveoli seen on transbronchial biopsy as well as in bronchoalveolar lavage fluid. The patient eventually also displayed a characteristic "crazy paving" pattern on radiographic imaging. Granulocyte macrophage-colony stimulating factor antibodies were negative, consistent with secondary PAP. PAP is a rare interstitial lung disease with only a few reported cases occurring after lung transplantation. The etiology is thought to be related to a defect in macrophage function caused by immunosuppression. Reduced immunosuppression has been associated with stabilization, but not reversal, of the condition in the case reported here. PAP is an exceptionally rare cause of dyspnea and radiographic infiltrates after lung transplantation and may be related to toxicity of immune-suppressive medications.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California 90095-1690, USA.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Albores
authors.forename: Atsuko
authors.initials: A
authors.lastname: Seki
authors.forename: Michael C
authors.initials: MC
authors.lastname: Fishbein
authors.forename: Fereidoun
authors.initials: F
authors.lastname: Abtin
authors.forename: Joseph P
authors.initials: JP
authors.lastname: Lynch
authors.forename: Tisha
authors.initials: T
authors.lastname: Wang
authors.forename: S Samuel
authors.initials: SS
authors.lastname: Weigt
mesh_terms: D001706:Biopsy; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D004417:Dyspnea / Q000209:etiology; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000601:surgery*; D007165:Immunosuppression Therapy / Q000009:adverse effects / Q000379:methods; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D016040:Lung Transplantation / Q000379:methods*; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*
chemical_list: D007166:Immunosuppressive Agents